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Malignant hyperthermia (MH) is a rare, often life-threatening complication of general anaesthesia. The timely diagnosis of an MH crisis in onset, as a prerequisite for successful therapy, can be difficult for the anaesthetist because of the few and non-specific early symptoms. This is even more so in patients in whom anaesthesia with MH trigger substances has already been performed in the past without any particular complications so leading to a false sense of security with regard to MH sensitivity. The case presented is a healthy young man with a congenital cleft lip, jaw and palette who developed a fulminant MH crisis during his ninth general anaesthesia. Post-operative research into the course of the previous anaesthesias revealed signs of MH crises which however proceeded abortively and were therefore unnoticed. In the case presented, the diagnosis was additionally complicated by the untypical course of the early symptoms. Tachycardia which in 80 % of cases is described as the first symptom of an MH crisis in onset, was at first completely absent and was only moderately pronounced in the full clinical picture of MH. On the other hand, a steady increase in body temperature, a cardinal symptom which usually appears later, was registered early. The suspected diagnosis of MH was then finally confirmed in the fourth hour after start of anaesthesia on the basis of the repeatedly increased end expiratory CO(2) levels. These could not otherwise be explained although several respiratory corrections were performed. Despite immediate MH specific therapy, the crisis developed in the following hour into the full clinical picture: maximum temperature of 41.4 degrees C, end expiratory CO(2) 100mm Hg, consumptive coagulopathy, acute renal failure and shock (systolic blood pressure < 50mm Hg, heart rate 115/minute). After 2 hours of specific intensive therapy, the patient was finally stabilized and transfer to the intensive care unit was possible. 24 hours after the event, the patient was could be extubated without any complications and 2 days later, he was transferred to the normal ward. The intra-operative diagnosis of MH was confirmed 3 days later by means of genetic analysis. Two mutations of the RYR1 gene were identified.

We report on a 25-year old ASA physical status I patient, who developed within 20 minutes a full-blown malignant hyperthermia (MH) in the context of a living donor liver transplantation after 180 minutes of uneventful anaesthesia. The only trigger substance applied was Sevoflurane. The patient had already received a short, uneventful anaesthesia with Isoflurane a couple of years ago. In the context of the special constellation an initial dose of Dantrolene of 10 mg/kg body weight was administered. The patient was stabilised within 30 minutes, and the enzyme levels remained low compared with other case reports. The post-operative in vitro caffeine halothane contracture testing confirmed that son and mother were susceptible to MH, contracture testing in the father was negative. All known triggers may cause life-threatening MH crisis - even after hours and after inconspicuous multiple exposures to known trigger substances. Therefore all trigger substances must be avoided in all patients susceptible to MH.

Hyperthermia, skeletal muscle rigidity, rhabdomyolysis, acidosis and multiple system insufficiency characterize malignant hyperthermia. Anaesthetic malignant hyperthermia follows halogenated volatile agents and/or depolarizing muscle relaxants utilization. Diagnosis is based on in vitro muscle contracture in response to halothane and/or caffeine exposure. Neuroleptic malignant syndrome affects patients taking neuroleptic drugs; clinical findings include hyperthermia, extrapyramidal rigidity, acidosis, neurovegetative instability and neurological signs. We report three neuroleptic malignant syndrome patients with positive muscle contracture tests which shows that muscle from neuroleptic malignant syndrome patients may in some instances show alterations similar to those of anaesthetic malignant hyperthermia.

The authors report on a course of malignant hyperthermia (MH) in an almost 5-years old boy. In the past, he had been anaesthetized two times with halothane without complications. The causative triggering agent was sevoflurane, a new user-friendly substance for paediatric anaesthesia. Forty five minutes after induction of anaesthesia he developed symptoms of a MH-crisis with increase in endexspiratory CO2 up 87 mmHg and followed by an increase in heart rate up to 160 beats/minute. The blood gas analysis showed a respiratory and metabolic acidosis. The timely administration of dantrolene rapidly reversed the life-threatening signs and prevent progression of the disease. It is apparent that monitoring of endtidal carbon dioxide by means of capnometry is of crucial importance in detecting MH at an early stage, and appropriate treatment is being instituted more promptly. By such early recognition, and treatment with dantrolene, we can reasonably except a further decrease in mortality and morbidity of this enigmatic disorder.

Since muscle relaxants have been implicated in triggering malignant hyperthermia (MH) in MH-susceptible humans and animals, the potential of new muscle relaxants for triggering MH needs to be assessed in vivo in MH-susceptible pigs. The triggering potential of atracurium was evaluated in six MH-susceptible pigs during one hour infusion of a 90% blocking dose (0.4 mg/kg/h) of atracurium. The mean recovery time (25% to 75%) was 10.2 min and a slight increase in heart rate (142 to 170 beats per minute) was observed. Rectal temperature decreased slightly (36.4 to 35.6 degrees C) and end tidal CO2 was stable at 5.46 kpa. In this study atracurium did not trigger MH in susceptible pigs.

We report on the fulminant crisis of malignant hyperthermia occurring in a 30-year-old female during kidney transplantation. In the past, she had been anaesthetised repeatedly without complications. Anaesthesia was induced with thiopental and vecuronium and continued with isoflurane/N2O/O2. After an initially normal course of anaesthesia, the patient developed symptoms of a fulminant malignant hyperthermia (MH) including excessive increase in end expiratory CO2, hyperkalaemia, tachycardia and hyperpyrexia. The patient was saved by the timely administration of dantrolene. A surgical revision required the next day because of bleeding was done under dantrolene cover and took an uncomplicated course. The patient was extubated 7.5 hours after the second intervention and transferred to a normal ward after 4 days. A subsequently performed in vitro contracture test clearly revealed susceptibility to malignant hyperthermia.

Besides offering a concise recapitulation of known facts, experiences and related viewpoints concerning malignant hyperthermia (MH), the present article offers a review of the most relevant new aspects in this field. Special emphasis is on genetics and pathogenesis of MH. The contents of the review are as follows: History; Definition of MH; Epidemiological aspects; Inheritance; Molecular genetics; Pathogenesis; Triggering agents; Awake Triggering; Sympathetic nervous system; Serotoninergic system; Involvement of other organs and cell systems; Clinical symptoms and diagnosis; MH and myopathies; Associated disorders; Treatment; Prophylaxis; Identification of susceptibility; MH testing centres in the FRG; Hot-line for MH emergencies

There are recent reports that inositol phosphate metabolism is involved in the development of malignant hyperthermia (MH). Consequently, we investigated the basal concentration of inositol phosphate products in skeletal and heart muscles of malignant hyperthermia-susceptible (MHS) and healthy control (MHN) swine. Different inositol phosphates were measured by high pressure liquid chromatography, including inositol trisphosphate, tetrakisphosphate, pentakisphosphate and hexakisphosphate. All inositol phosphate products measured had a higher concentration in MHS than MHN in skeletal (304-1330%) as well as heart muscles (134-440%). An activation of the inositol phosphate metabolism has been shown to mobilise intracellular calcium from the sarcoplasmic reticulum. It is therefore concluded that, firstly, besides involvement of the skeletal muscles a primary myocardial abnormality in MHS is possible; and secondly, the idea that the inositol phosphate metabolism could be involved in the development of MH is additionally supported.

The essential epidemiologic, pathophysiologic and clinical data of malignant hyperthermia are presented. Prophylactic measures, early recognition and an effective therapy schedule may reduce the appallingly high lethality of this rare complication during general anaesthesia.

Narcots and muscle relaxants are proven causes of malignant hyperthermia. Alcohol and a large number of drugs are capable of inducing myopathic changes which resemble malignant hyperthermia. The case of a 47-year-old man is reported who presented with the clinical symptoms of maligant hyperthermia. The similarity in the course of the disturbance, the clinical and chemical findings and the changes in the morphological features of the muscle suggested that the abnormally high body temperature had been induced by psychotropic durgs in a pre-disposed patient with an history of chronic alcoholism.

Two cases of anaesthesia-induced malignant hyperthermia are reported. One patient died; the other, a 29-years-old woman, recovered without ascertainable after-effects although high temperature persisted for a relatively long time. Suggestions for the early diagnosis of malignant hyperthermia are analysed and the problem of procaine dosage is briefly discussed.

Malignant hypothermia seems to depend on a latent hereditary disposition, in the couse of which a defect in the storage of calcium at the cell membrane of the skeletal - and heart muscle happens. The aetiology of the disease could not be fully explained until now. The mechanism is especially triggered by medicaments in routine anaesthesiological use. There is no preanaesthesiologic method to detect persons prone to malignant hypothermia. 3 cases of this kind are dealt with in detail. All 3 patients came to death although the measures of treatment taken immediately corresponded completely to the present state of knowledge.

A statistical review is given on cases of malignant hyperthermia in Hamburg. The total number of general anesthetics from 1970--1976 was 960 000. 10 cases of malignant hyperthermia have been observed so that a crude estimate of the incidence would be about 1 : 100 000. Some epidemiological aspects of the patients in Hamburg are discussed.

A 24-year-old chronic alcoholic survived malignant hyperthermia which developed in connection with maxillo-facial operation. Electron-microscopic investigations of muscle biopsies disclosed so called tubular aggregates. The possible relations between these structures and the occurrence of malignant hyperthermia on the basis of an alcoholic myopathy are discussed. People suffering from idiopathic recurrent myoglobinuria have to be regarded as patients-at-risk.